Can Polycythemia Vera Cause a Positive ANA?
No, polycythemia vera (PV) does not cause a positive antinuclear antibody (ANA) test—ANA positivity is not a recognized feature or diagnostic criterion of PV.
Why ANA is Not Associated with PV
The diagnostic criteria and clinical features of PV are well-established and do not include autoimmune markers like ANA:
PV diagnostic criteria focus on hematologic parameters and JAK2 mutations, not autoimmune markers. The WHO 2016 criteria require elevated hemoglobin/hematocrit, bone marrow hypercellularity with trilineage growth, and JAK2 V617F or exon 12 mutations 1, 2.
Characteristic clinical features of PV include erythrocytosis, aquagenic pruritus (48% of patients), splenomegaly, thrombocytosis, leukocytosis, and microvascular disturbances such as headaches and visual changes 2, 3. None of these involve autoimmune phenomena that would trigger ANA production.
Laboratory abnormalities in PV include low serum erythropoietin levels (>90% specificity), elevated leukocyte alkaline phosphatase, and increased vitamin B12 levels 1, 4—but autoimmune markers are conspicuously absent from all diagnostic algorithms and disease descriptions 1, 2, 4.
Clinical Implications
If a patient with PV has a positive ANA, consider this a coincidental finding or investigate for a separate autoimmune condition 2, 5. The prevalence of positive ANA in the general population (particularly low-titer positivity) means overlap can occur by chance.
Do not attribute autoimmune symptoms to PV itself—PV causes thrombotic complications (arterial thrombosis in 16%, venous thrombosis in 7%), bleeding risk with extreme thrombocytosis, and progression to myelofibrosis (12.7%) or acute myeloid leukemia (6.8%) 3, 5, but not autoimmune manifestations.
The diagnostic workup for PV should focus on JAK2 mutation testing (positive in >95% of cases), serum erythropoietin measurement, and bone marrow examination when indicated 2, 4, 5—not on autoimmune serologies.